2027 ICD-10-CM Diagnosis Code D81.1Severe combined immunodeficiency [SCID] with low T- and B-cell numbers

ICD-10-CM Codes›D50–D89›D80-D89›D81

ICD-10-CM D81.1
CMSSource: CMS FY 2027 ICD-10-CM dataset · Effective Oct 1, 2026 – Sep 30, 2027

D81.1 is a billable ICD-10-CM diagnosis code for severe combined immunodeficiency [SCID] with low T- and B-cell numbers. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 808 through 810. As a secondary diagnosis, it counts as a complication or comorbidity (CC) and moves an inpatient stay to a higher severity level within its MS-DRG family. It does not count, however, when the principal diagnosis is one of 44 closely related codes. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Immunity disorders.

For Medicare Advantage risk adjustment, D81.1 maps to CMS-HCC Category 114 (Common Variable and Combined Immunodeficiencies) under the V28 model, adding a risk factor of about 2.262 for a community, non-dual, aged beneficiary in payment year 2027.

Code Identity

ICD-10-CM Code
D81.1
Billable Status
Yes — Valid for Submission
Code Describes
Severe combined immunodeficiency [SCID] with low T- and B-cell numbers
Short Description
Severe combined immunodeficiency w low T- and B-cell numbers
Parent Code
Combined immunodeficiencies

Code Classification

ChapterD50–D89Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
SectionD80-D89Certain disorders involving the immune mechanism
CategoryD81Combined immunodeficiencies
This CodeD81.1Severe combined immunodeficiency [SCID] with low T- and B-cell numbers

Medicare Risk Adjustment (HCC)Billing

D81.1 maps to a payment category in the CMS-HCC model used to risk-adjust Medicare Advantage payments. Weights are the published community factors for payment year 2027.

CMS-HCC V28 Category (Payment Model)
HCC 114— Common Variable and Combined Immunodeficiencies
Payment HCC · PY 2027 one of 18 ICD-10-CM codes in this category
Risk Adjustment Factor (RAF) Weight
+2.262
community, non-dual, aged · ranges 0.691–2.789 across segments
Hierarchy
Supersedes HCC 115
less severe related categories are not paid alongside HCC 114
Prior Model (CMS-HCC V24)
HCC 47
V24 retired V28 pays 100% of MA risk scores since PY 2026
Other CMS Models
PACE (CMS-HCC V22): HCC 47 · ESRD (V21): HCC 47 · ESRD (V24): HCC 47
ESRD V21 weights: 0.097 dialysis, 0.549–0.688 functioning graft · ESRD V24 weights: 0.078 dialysis, 0.523–0.803 functioning graft
Part D (RxHCC)
RxHCC 99 — Immune Disorders
also risk-adjusts in the Part D prescription drug model (V08)

Source: CMS Payment Year 2027 risk adjustment mappings and model software. Weights are relative factors, not dollar amounts; a beneficiary's total RAF also includes demographics and interactions. Browse all CMS-HCC categories.

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Autosomal dominant T-cell negative, B-cell negative severe combined immunodeficiency due to activated RAC2 defect
  • Combined immunodeficiency due to partial RAG1 deficiency
  • Disorder of eosinophil
  • Hereditary eosinophilia
  • Omenn syndrome
  • SCID due to absent peripheral T cell maturation
  • SCID due to absent T cell receptor
  • SCID production
  • SCID due to absent IL-2 receptor
  • Severe combined immunodeficiency due to complete RAG1 and/or RAG2 deficiency
  • Severe combined immunodeficiency due to CTPS1 deficiency
  • Severe combined immunodeficiency due to LCK deficiency
  • Severe combined immunodeficiency with low T- and B-cell numbers

Instructional NotesGuidance

Instructions from the official ICD-10-CM Tabular List that apply to D81.1: its own notes plus those printed at D81, block D80-D89, and Chapter 3. A note printed at a category, block or chapter applies to every code under it.

Includes

Further defines, or gives examples of, the content of the category.

From Block D80-D89 Certain disorders involving the immune mechanism applies to 83 codes
  • defects in the complement system
  • immunodeficiency disorders, except human immunodeficiency virus [HIV] disease
  • sarcoidosis

Excludes1

Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.

From D81 Combined immunodeficiencies applies to 17 codes
  • autosomal recessive agammaglobulinemia (Swiss type) (D80.0)
From Block D80-D89 Certain disorders involving the immune mechanism applies to 83 codes
  • autoimmune disease (systemic) NOS (M35.9)
  • functional disorders of polymorphonuclear neutrophils (D71-)

Excludes2

Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.

From Block D80-D89 Certain disorders involving the immune mechanism applies to 83 codes
  • human immunodeficiency virus [HIV] disease (B20)
From Chapter 3 (D50-D89) Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism applies to 324 codes
  • autoimmune disease (systemic) NOS (M35.9)
  • certain conditions originating in the perinatal period (P00-P96)
  • complications of pregnancy, childbirth and the puerperium (O00-O9A)
  • congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)
  • endocrine, nutritional and metabolic diseases (E00-E88)
  • human immunodeficiency virus [HIV] disease (B20)
  • injury, poisoning and certain other consequences of external causes (S00-T88)
  • neoplasms (C00-D49)
  • symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)

Source: CMS ICD-10-CM Tabular List. How to read instructional notes.

Referenced in Other NotesGuidance

Instructional notes printed at other codes that name D81.1, its category, or a range that includes it.

Excludes1 3

These codes carry an Excludes1 note naming D81.1: they are not reported together with it, unless the two conditions are unrelated.

  • E79 Disorders of purine and pyrimidine metabolism
    combined immunodeficiency disorders (D81.-) names D81.-, which includes this code
  • D72 Other disorders of white blood cells
    immunity disorders (D80-D89) names D80-D89, which includes this code
  • D84.81 Immunodeficiency due to conditions classified elsewhere
    certain disorders involving the immune mechanism (D80-D83, D84.0, D84.1, D84.9) names D80-D83, which includes this code

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR BLD008
Immunity disorders
Default principal diagnosis: inpatient Yes · outpatient Yes

Patient EducationClinical

Immune System and Disorders

Your immune system is a complex network of cells, tissues, and organs. Together they help the body fight infections and other diseases.

The full article covers:

  • What is the immune system?
  • What are the parts of the immune system?
  • How does the immune system work?
  • What are the types of immunity?
  • What can go wrong with the immune system?

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert D81.1 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
279.2 Combined immunity defic
Approximate The match is approximate rather than exact.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2026No changes
FY 2027CurrentCurrent code set, no changesEffective October 1, 2026 through September 30, 2027.

Questions About D81.1Overview

What is the ICD-10 code for severe combined immunodeficiency [SCID] with low T- and B-cell numbers?

The ICD-10-CM code for severe combined immunodeficiency [SCID] with low T- and B-cell numbers is D81.1 (sometimes written as D811). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

Is D81.1 (Combined immunodeficiencies) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report severe combined immunodeficiency [SCID] with low T- and B-cell numbers on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

What MS-DRG does D81.1 group to?

When severe combined immunodeficiency [SCID] with low T- and B-cell numbers is the principal diagnosis on an inpatient stay, it groups to MS-DRG 808, 809, 810, with relative weights from 1.0749 to 2.1706 depending on complications. Higher weights mean higher Medicare reimbursement.

Is D81.1 a CC or MCC?

CMS lists D81.1 as a CC (complication or comorbidity) for FY 2027. Reported as a secondary diagnosis, it moves the inpatient stay to a higher-weighted DRG within its severity family. It does not count when the principal diagnosis is one of the 44 closely related codes in its exclusion list.

Can D81.1 be reported with D80.0?

Not as a rule. The Excludes1 note at D81, which applies to D81.1, lists "autosomal recessive agammaglobulinemia (Swiss type) (D80.0)". An Excludes1 note means the excluded code is never reported together with this one; the only exception is when the two conditions are unrelated to each other (Official Guidelines, Section I.A.12.a).

What HCC is D81.1?

D81.1 (severe combined immunodeficiency [SCID] with low T- and B-cell numbers) maps to CMS-HCC Category 114 (Common Variable and Combined Immunodeficiencies), commonly written as HCC 114, in the CMS-HCC V28 model used for Medicare Advantage risk adjustment in payment year 2027. It mapped to HCC 47 under the retired V24 model. It also maps in the PACE (CMS-HCC V22), ESRD (V21), and ESRD (V24) models. In the Part D prescription drug model it maps to RxHCC 99.

Does D81.1 risk-adjust for Medicare Advantage payment?

Yes. When documented and reported on a Medicare Advantage encounter, D81.1 adds a risk adjustment factor of about 2.262 to the beneficiary's RAF score for a community, non-dual, aged enrollee (published V28 weights range from 0.691 to 2.789 depending on the payment segment). HCC 114 sits at the top of its hierarchy, so no other condition category supersedes it. See the full factor table on the HCC 114 category page.